Idiopathic Mast Cell Activation Syndrome With Associated Salicylate Intolerance

• Main Authors: Tobias Rechenauer, Martin Raithel, Thomas Götze, Gregor Siebenlist, Aline Rückel, Hanns-Wolf Baenkler, Arndt Hartmann, Florian Haller, André Hoerning
• Format: Article
• Language: English
• Published: Frontiers Media S.A. 2018-03-01
• Series: Frontiers in Pediatrics
• Subjects: idiopathic mast cell activation syndrome; mast cell activation; salicylate intolerance; mastocytosis; endoscopically guided lavage; abdominal pain
• Online Access: http://journal.frontiersin.org/article/10.3389/fped.2018.00073/full

Idiopathic mast cell activation syndrome can be a rare cause for chronic abdominal pain in children. It remains a diagnosis by exclusion that can be particularly challenging due to the vast variety of possible clinical manifestations. We present a 13-year-old boy who suffered from a multitude of unspecific complaints over a long period of time. In this case, an assessment of mast cell-derived metabolites and immunohistochemical analysis of bioptic specimen was worthwhile. After ruling out, primary (oncologic) and secondary causes for mast cell activation, pharmacologic treatment adapted to the patient’s salicylate intolerance resulted in a major relief of symptoms.